21 research outputs found
Linking Outdoor Recreation and Economic Development: A Feasibility Assessment of the Obed Wild and Scenic River, Tennessee
Mouse and computational models link Mlc2v dephosphorylation to altered myosin kinetics in early cardiac disease
Education Policy and Welfare Regimes in OECD Countries: Social Stratification and Equal Opportunity in Education
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The Prevalence of Retinal Disease and Associated Central Nervous System Disease in Young Patients with Incontinentia Pigmenti
To evaluate the prevalence of retinal disease on fluorescein angiography (FA) in patients with incontinentia pigmenti (IP) and to compare the severity of retinal disease in those with and without known central nervous system (CNS) disease.
Multi-institutional consecutive retrospective case series
New patients with a diagnosis of IP seen at the Casey Eye Institute, Oregon Health and Science University, Moran Eye Center, University of Utah, Wills Eye Hospital, or Bascom Palmer Eye Institute, University of Miami from December 2011 to September 2018.
Detailed ophthalmoscopic examination and FA were recommended to all new patients and performed on every patient who had parental consent. Ophthalmoscopic findings and FA images were graded for severity by two masked graders on a 3-point scale: 0 = no disease, 1 = vascular abnormalities without leakage, 2 = leakage or neovascularization, 3 = retinal detachment. Presence of known CNS disease was documented. Additional cases were obtained from a pediatric retina listserv for examples of phenotypic variation.
Proportion of eyes noted to have disease on ophthalmoscopy compared with FA. Severity of retinal disease in those with and without known CNS disease.
Retinal pathology was detected in 18/35 (51%) by indirect ophthalmoscopy and 26/35 (74%) by FA (p=0.048) in a predominantly pediatric population (median age = 9 months). Ten patients (29%) had known CNS disease at the time of the eye exam. A Wilcoxon ranked sums test indicated that the retinal severity scores for patients with CNS disease (median = 2) were significantly higher than the retinal severity scores for patients without CNS disease (median = 1), z = -2.12, p = 0.034.
Retinal disease is present in the majority of patients with IP, and the ophthalmoscopic examination is less sensitive than FA for detection of disease. There may be a correlation between the severity of retinal and CNS disease.
Retinal disease was detected in >70% of consecutive patients with incontinentia pigmenti from 3 centers where fluorescein angiography is recommended on all patients. Patients with known central nervous system disease exhibited more severe retinal pathology